SS is a prevalent and underdiagnosed systemic disease that primarily affects epithelial tissue. It may affect renal function either as epithelial disease causing tubulointerstitial nephritis or as an immune complex-mediated glomerulopathy. These lesions may cause a variety of clinical features, both overt and occult. The epithelial disease is mediated by B and T cells, notably the Th17 subtype. We review the prevalence of renal SS, its presentation, likely pathogenesis and treatment.
Keywords: B cells; Fanconi syndrome; Sjögren’s syndrome; Th17 cells; autoantibodies; autoimmune epithelialitis; distal renal tubular acidosis; hypocomplementaemia; tubulointerstitial nephritis; vasculitis.
© The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.